Dystroglycan and muscular dystrophies related to the dystrophin-glycoprotein complex
. Ann Ist Super Sanita 2003;39:173-81.
The relevance of the dystrophin-glycoprotein complex
in cardiomyopathy has been demonstrated in mouse models of sarcoglycanopathies, in which mutations in the [beta], [delta], or [gamma] sarcoglycan genes produce both muscular dystrophy and dilated cardiomyopathy that reproduce the phenotype of the BIO-TO2 strain.
It binds to the dystrophin-glycoprotein complex that interacts with both contractile proteins and signaling proteins for protein synthesis such as phosphatidylinositol 3-kinase (1,8,32).
Tensile stimuli that exceed the plastic phase may cause micro-lesions and sarcolemmal damage, which can impair the integrity of dystrophin and the dystrophin-glycoprotein complex, resulting in an increase of permeability to ions and small molecules and lead to muscle degeneration and necrosis.
ECM proteins, such as laminin, bind to both membrane proteins and the dystrophin-glycoprotein complex (2).
Petrof, "Molecular pathophysiology of myofiber injury in deficiencies of the dystrophin-glycoprotein complex
," American Journal of Physical Medicine and Rehabilitation, vol.
Rando, "The dystrophin-glycoprotein complex
, cellular signaling, and the regulation of cell survival in the muscular dystrophies," Muscle & Nerve, vol.
Dystrophin connects to other glycoproteins to form the dystrophin-glycoprotein complex (DGC).
The dystrophin-glycoprotein complex in the prevention of muscle damage.